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Rheumatology > Connective Tissue Diseases > Flashcards

Connective Tissue Diseases Flashcards

1
Q

What is Systemic Lupus Erythematous (SLE)

A

A chronic autoimmune (seropositive) disease that can affect almost any organ system, therefore presentation can be very variable

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2
Q

Autoantibodies associated with SLE

A

ANA (99% sensitive, but not specific)
Anti-DNA (70% sensitive, but 99% specific)
Anti-Sm (33% sensitive, 99% specific)

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3
Q

main organs/systems affected by SLE

A 
skin 
joints 
kidneys 
blood cells 
nervous system
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4
Q

what type of hypersensitivity reaction is SLE

A

Type III (immune complex)

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5
Q

immunological response in SLE

A

Anti-DNA antibodies and ANA antibodies form immune complexes with antigens which are deposited in small vessels in skin, joints, kidneys, resulting in complement activation

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6
Q

what is the cause of SLE

A

largely unknown, but it is multifactorial

i.e. environmental insult in a genetically predisposed individual

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7
Q

is SLE more common in males or females?

Typical age of onset?

A

Females! >90%

age of onset 20s-30s

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8
Q

constitutional symptoms in SLE

A

fever
malaise
weight loss

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9
Q

MSK symptoms in SLE

A

arthralgia (joint pain)
myalgia (muscle pain)
inflammatory arthritis - non-erosive
increased prevalence of AVN esp. femoral head

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10
Q

Muco-cutaneous symptoms in SLE

A 
malar butterfly rash 
photosensitivity 
discoid lupus 
subacute cutaneous lupus 
oral/nasal ulceration 
Raynaud's phenomenon 
sclerodactyly 
alopecia
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11
Q

Renal symptoms in SLE

A

lupus nephritis

persistent proteinuria

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12
Q

Respiratory symptoms in SLE

A 
pleurisy 
pleural effusions
pneumonitis 
pulmonary embolism 
pulmonary hypertension 
interstitial lung disease
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13
Q

Haematological symptoms in SLE

A

leukopenia
lymphopenia
anaemia
thrombocytopenia

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14
Q

Neurological symptoms in SLE

A

seizures
psychosis
headache
aseptic meningitis

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15
Q

Cardiac symptoms in SLE

A 
pericarditis 
pericardial effusion 
pulmonary hypertension 
sterile endocarditis 
accelerated ischaemic heart disease
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16
Q

GI symptoms in SLE

A

autoimmune hepatitis
pancreatitis
mesenteric vasculitis

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17
Q

Ix for SLE

A

FBC - anaemia, leukopenia, thrombocytopenia

Immunology -
ANA - sensitive, but not specific. Take seriously if other markers are also positive
Anti-DNA - specific, but only +ve in 60%

Urinalysis -
glomerulonephritis

Imaging -
evidence of organ involvement
CT, MRI, echo

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18
Q

General approach to Tx of SLE

A

Depends on its manifestations

counselling, regular monitoring, avoidance of excessive sun exposure, pregnancy issues (often flares)

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19
Q

Tx of skin disease and arthralgia in SLE

A
  1. Hydroxychloroquine (DMARD)
  2. NSAIDs (naproxen) - for as short a period as poss
  3. Corticosteroids (pred)
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20
Q

Tx of SLE if there is inflammatory arthritis or organ involvement

A
  1. Immunosuppression (azothioprine)
  2. DMARDs
  3. Corticosteroids (pred)
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21
Q

Tx of SLE if there is lupus nephritis or CNS involvement

A
  1. Cyclophosphamide (alkylating chemo agent)

2. IV steroids

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22
Q

Tx of SLE in unresponsive cases

A

biologic therapy - Rituximab

IV immunoglobulins

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23
Q

How should SLE be monitored

A

Check anti-DsDNA antibodies and complement levels regularly

Check urinalysis for blood or protein

Cardio - BP and cholesterol

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24
Q

What is Sjogrens syndrome

A

Autoimmune seropositive condition that is characterised by lymphocytic infiltrates in exocrine glands

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25
Q

What is an exocrine gland

A

glands that produce and secrete substances onto an epithelial surface by way of a duct

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26
Q

What glands are usually affected in Sjogren’s syndrome

A

Lacrimal –> therefore dry eyes (keratonconjunctivitis sicca)

salivary –> therefore dry mouth (xerostomia)

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27
Q

Apart from dry eyes and mouth, what other symptoms can be present in Sjogrens syndrome

A 
arthralgia 
fatigue 
vaginal dryness
parotid gland swelling 
peripheral neuropathy 
interstitial lung disease
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28
Q

Autoantibodies associated with Sjogrens syndrome

A

Anti-Ro

Anti-La

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29
Q

Does Sjogren’s syndrome always occur on its own

A

No!

Can be primary Sjogren’s syndrome (no other conditions)

Or secondary Sjogren’s syndrome - to other autoimmune conditions e.g. RA, SLE

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30
Q

Test for ocular dryness in Sjogrens

A

Schirmer’s test

quantitative measure of tears. filter paper placed in lower conjunctival sac. test is +ve is <5mm of paper is wet after 5 mins.

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31
Q

Tx of Sjogren’s syndrome

A

Dry eyes - luricating eye drops

Dry mouth - artificial saliva, pilocarpine (cholinergic)

Arthralgia and fatigue - hydroxychloroquine

Severe systemic disease - immunosuppression

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32
Q

What is systemic sclerosis also known as

A

scleroderma

33
Q

What is systemic sclerosis

A

a mulit-system seropositive autoimmune disease characterised by functional and structural abnormalities of small blood vessels, fibrosis of skin and internal organs.

34
Q

2 main subtypes of systemic sclerosis & auto-antibodies associated with each

A

limited systemic sclerosis - anti-centromere antibody

diffuse systemic sclerosis - anti Scl-70 antibody

35
Q

What is the difference between limited and diffuse systemic sclerosis

A

more rapid development of skin changes and earlier organ involvement in diffuse systemic sclerosis compared to limited systemic sclerosis

36
Q

cutaneous presentations of systemic sclerosis

A

(skin eventually becomes thickened and tightened)

RAYNAUDS!!

centrally located skin sclerosis on arms, faces and/or neck

scerodactyly (thickening of skin in fingers and toes)

telangiectasia (red dots on the fingers, palms, face)

calcinosis (subcutaneous deposits of calcium in digits)

37
Q

organ presentations of systemic sclerosis

A 
pulmonary hypertension 
pulmonary fibrosis 
accelerated hypertension 
eventual renal crisis 
inflammatory arthritis 
myositis 
gut involvement - presents as dysphagia, malabsorption, bacterial overgrowth
38
Q

pneumonic to remember presentation of limited systemic sclerosis

A

CREST syndrome

Calcinosis 
Raynaud's 
Esophageal &amp; gut dysmotility 
Sclerodactyly 
Telangiectasia
39
Q

Mx of renal crisis in scleroderma

A
  1. ACEi - lisinopril
  2. additional anti-hypertensives
  3. renal dialysis or transplant
40
Q

Mx of raynaud’s in scleroderma with no digital ulceration

A
  1. CCB - amlodipine, nifedipine

2. aspiriin or Pentoxifylline

41
Q

Mx of Raynauds in scleroderma with digital ulceration

A
  1. phosphodiesterase 5 inhibitor - sildenafil, bostentan

2. Iloprost

42
Q

Mx of skin involvement in scleroderma

A
  1. topical immollient

pruritus - steroids

thickening - cyclophosphamide

43
Q

Mx of GI involvement in scleroderma

A

PPI - omeprazole

44
Q

Mx of interstitial lung disease in scleroderma

A

cyclophosphamide

45
Q

what is Mixed Connective Tissue Disease (MCTD)

A

an overlap syndrome - a group of conditions that meets the classification criteria for more than 1 well recognised rheumatological disease

46
Q

Features of MCTD

A 
Raynauds 
Arthralgia/arthritis 
Myositis 
Sclerodactyly 
Pulmonary hypertension 
Interstitial lung disease
47
Q

Auto-antibody associated with MCTD

A

Anti-RNP antibodies

48
Q

What is anti-phospholipid syndrome

A

an autoimmune disorder that manifests clinically as recurrent venous or arterial thrombosis and/or fetal loss.

49
Q

auto-antibodies associated with anti-phospholipid syndrome

A

Anticardiolipin antibodies

Lupus anticoagulant antibodies

50
Q

pneumonic to remember anti-phospholipid syndrome

A

CLOT

Coagulation defect
Livedo reticularis
Obstetric (recurrent miscarriage)
Thrombocytopenia

51
Q

What is the criteria for pregnancy loss in anti-phospholipid syndrome

A

Pregnancy loss with no other explanation 10-34/40 weeks or 3 pregnancy losses with no other explanation <10/40 weeks

52
Q

What is livedo reticularis

A

Lace-like purplish discolouration of skin seen in anti-phospholipid syndrome

53
Q

What valvular heart problem can be found in patients with Anti-Phospholipid syndrome

A

Sterile endocarditis –> Libman-Sacks endocarditis

embolisation can occur from these lesions

54
Q

Tx of anti-phospholipid syndrome

A

initial anti-coagulation in acute thrombosis –> LMWH
then life-long warfarin

pregnancy - LMWH

55
Q

do all patients with anti-phospholipid syndrome receive anti-coagulation

A

No - if they have it but have never had thrombosis, they don’t require anti-coagulation

56
Q

What is gout

A

a crystal arthropathy caused by deposition of URATE crystals within a joint

57
Q

what is uric acid

A

the final compound in the breakdown of purines in DNA metabolism (adenine and guanine).

58
Q

How does hyperuricaemia occur to cause gout

A

increased urate production e.g.
inherited enzyme defects
psoriasis
alcohol

decreased renal urate excretion e.g. 
chronic renal impairment 
volume depletion e.g. heart failure 
hypothyroidism 
thiazide diuretics
59
Q

most common joints affected by gout

A

first MTP joint
ankle
knee

60
Q

presentation of gout

A

intense red hot swollen joint

symptoms last for 7-10 days if untreated then resolve

61
Q

what are ‘gouty tophi’

A

painless white accumulation of uric acid which can occur in the soft tissues and occasionally erupt through the skin

62
Q

Ix for gout

A

synovial fluid sample for polarising microscopy

  • needle shaped crystal s
  • negative birefringence
63
Q

Tx acute gout

A
  1. NSAID
  2. Colchicine
  3. Corticosteroids
64
Q

Prophylaxis for gout

A

Allopurinol (xanthine oxidase inhibitor)

65
Q

What medication should NOT be given in acute gout

A

Allopurinol - can potentiate further flares

66
Q

When should prophylaxis for gout be started

A

2 weeks after acute attack

67
Q

Adverse effects of allopurinol

A

rash

azothioprine interaction

68
Q

What is pseudogout

A

a crystal arthropathy caused by deposition of CALCIUM PYROPHOSPHATE crystals in a joint

69
Q

What conditions are associated with pseudogout

A

hyperparathyroidism

amyloidosis

70
Q

Ix of pseudogout

A

synovial fluid sample for polarising microscopy

  • rhomboid shaped crystals
  • positive birefringement
71
Q

What is chondrocalcinosis

A

calcium pyrophosphate deposition in cartilage in the absence of acute inflammation

72
Q

Tx of pseudogout

A
  1. NSAIDs

2. Intra-articular steroids

73
Q

Prophylaxis for pseudogout

A

There is none!!

74
Q

low levels of which complement are associated with developing SLE

A

C3 & C4 (C4a and C4b)

75
Q

Drugs causing drug-induced lupus

A

procainamide (Na channel blocker)

hydralazine (anti-hypertensive)

76
Q

presentation of drug induced lupus

A

arthralgia
myalgia
skin (malar rash) and pulmonary involvement (pleurisy)
ANA +ve in 100%, Anti-DNA -ve

77
Q

radiological features of gout

A

joint effusion (early sign)
well-defined “punched out” lesions with sclerotic margins
preservation of joint space (not in late)
no periarticular osteopenia
soft tissue tophi

78
Q

pathogenesis of SLE

A

cellular remnants containing nuclear material are transferred to lymphatic tissues.
They are presented to T cells which then stimulates B cells to produce autoantibodies. IgG antibodies attack DNA resulting in antigen-antibody complexes causing damage in various areas

79
Q

pneumonic to remember the causes of gout

A

DART

Diuretics
Alcohol
Renal disease
Trauma

Rheumatology (9 decks)

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